Less than 500 cases of PG have been reported in the literature. Similarly, less than 200 cases of Good syndrome have been reported with an estimated prevalence of 1 in 500,000 people.1,5 PG typically presents with acute epigastric pain and malaise, which occurred in our patient, although with a subacute presentation. Upper endoscopy, the diagnostic gold standard, classically revealed a thickened and edematous gastric mucosa and, occasionally, purulent discharge which is pathognomonic. Primary and secondary forms have been described. Primary PG occurs after injury to the gastric mucosa, either following an endoscopic intervention or in the presence of invasive carcinoma.6 Secondary forms occur through adjacent/local (eg, pancreas) or hematogenous dissemination.1 Alternatively, PG can be classified by the extent of involvement (localized or diffuse). Diffuse PG has higher mortality rates (10 vs 54%).3,7 This case was classified as primary and diffuse.
Streptococcus pyogenes is isolated in approximately 70% of the cases, which contrasts with our case, where S. oralis was isolated.1 This is the first case of PG where this microorganism has been isolated. Early antibiotic therapy with appropriate stewardship is essential, as demonstrated in this report.1 Indication and timing of surgery represent a controversial therapeutic aspect of PG. Surgery has been associated with a reduced mortality rate (20% vs 50% in patients undergoing medical treatment).3 Thus, it should always be considered in patients with a lack of response and/or worsening despite conservative treatment.8 Our patient's stable clinical condition motivated conservative treatment but because of a suboptimal response, gastrectomy was indicated, leading to a favorable outcome.
Risk factors for PG include a history of gastrointestinal surgery, endoscopic procedures, hematologic malignant diseases (eg, leukemia), acquired and primary immunodeficiency disorders, alcoholism, and/or uncontrolled diabetes mellitus.1–3 Still, up to 50% of patients do not have a clearly identified risk factor.2,3 Good syndrome, the risk factor for PG in our patient, occurs in patients in their 50s and 60s, equally affecting both genders. Its pathogenesis remains unknown, although evidence suggests both primary bone marrow and/or T cell defect. Clinical presentation varies, and symptoms related to the thymoma, which may precede (up to 18 years) the diagnosis of Good syndrome in 42.4% of the cases, may be the first manifestations.9 Alternatively, patients may present with recurrent invasive and/or opportunistic infections, respiratory tract infections being the most common.4,5 In one case series, 90.5% of the patients debuted with invasive bacterial infections and 86% developed pneumonia; the most common bacteria were Streptococcus pneumoniae (33%) and Haemophilus influenzae (29%). Interestingly, concomitant autoimmune disease is present in 26%–76% of the patients, the most common being pure red cell aplasia, hypothyroidism, inflammatory arthritis, and myasthenia gravis, which is compatible with our case.5,10
According to case series and case reports, the laboratory findings in Good syndrome include hypogammaglobulinemia (100%), decreased B cells (87% of the cases), decreased CD4+ T cells (73.2%), and inverted CD4/CD8 ratio (76.1%), among others.9 Our patient presented with decreased levels of every IgG subclass, except IgG4, and low CD4+ T count. Good syndrome should be considered in patients older than 40 years with atypical, opportunistic, and/or recurrent infections, particularly those with a diagnosed thymoma. Importantly, thymectomy (and its extent), although an important indicator of long-term prognosis, does not reverse the immunological abnormalities. Thus, acknowledging the quality of the evidence, we recommend that every patient with a previous or current diagnosis of thymoma should have immunoglobulin levels measured.4,5
Treatment includes long-term immunoglobulin replacement therapy, which may decrease the number of recurrent infections. Patients with Good syndrome have an overall survival rate of 70% at 5 years, whereas other primary immunodeficiencies, such as common variable immunodeficiency or X-linked agammaglobulinemia, have survival rates of almost 100% at 5 years.4
In conclusion, we report the first case of a patient with Good syndrome complicated with PG. Early and aggressive treatment of patients with PG should be the norm, always considering surgery in severe cases and/or in those failing conservative treatment. Risk factors, such as underlying immunodeficiencies, should be conscientiously investigated.
Author contributions: All authors contributed equally to this manuscript. A. Campos-Murguía is the article guarantor.
Financial disclosure: BA Marfil-Garza is currently being supported by a joint stipend by the Fundación para la Salud y la Educación Salvador Zubirán and the Consejo Nacional de Ciencia y Tecnología.
Informed consent was obtained for this case report.
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© 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.
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